Abstract

Objective

The purpose of this study was to prospectively analyze the outcomes of patients with anomalous aortic origin of a coronary artery undergoing surgical intervention according to a standardized management algorithm.

A total of 44 patients, with a median age of 14 years (8-18 years), underwent surgical intervention: 9 (20%) for the anomalous left coronary artery and 35 (80%) for the anomalous right coronary artery. Surgical procedures included unroofing in 35 patients (80%), translocation in 7 patients (16%), ostioplasty in 1 patient (2%), and side-side-anastomosis in 1 patient (2%). One patient who presented with aborted sudden cardiac death from an anomalous left coronary and underwent unroofing presented 1 year later with a recurrent episode and was found to have an unrecognized myocardial bridge and persistent compression of the coronary requiring reintervention. At last follow-up, 40 patients (91%) are asymptomatic and 4 patients have nonspecific chest pain; 42 patients (95%) have returned to full activity, and 2 patients are awaiting clearance.

Conclusions

Surgical treatment for anomalous aortic origin of a coronary artery is safe and should aim to associate the coronary ostium with the correct sinus, away from the intercoronary pillar. After surgery, the majority of patients are cleared for exercise and remain asymptomatic. Longer follow-up is needed to assess the true efficacy of surgery in the prevention of sudden cardiac death.